PDF(Pubmed)
Abstract：
Central nervous system involvement in primary Sjögren\'s syndrome (pSS) is less common and usually presents as white matter lesions, neuromyelitis optica spectrum disorder (NMOSD), or transverse myelitis. NMOSD is an immune-mediated inflammatory demyelinating disease of the central nervous system with a high rate of relapse and significant disability. Studies have shown that patients with pSS combined with NMOSD have more severe symptoms and poorer prognosis. Here, we present a case of critical illness in pregnancy-associated NMOSD combined with Sjögren\'s syndrome. The patient was a 30-year-old pregnant woman with a history of Sjögren\'s syndrome who was diagnosed with NMOSD. She received combination therapy with steroids, intravenous immunoglobulin (IVIG), and hydroxychloroquine during pregnancy, resulting in partial resolution of numbness below the waist. However, due to irregular medication adherence outside the hospital setting, she developed weakness in her right lower limb accompanied by inability to move it, while her left lower limb still had some mobility but occasional numbness along with urinary and fecal incontinence. Ten days later, she was admitted to the emergency department where an emergency cesarean section was performed to deliver a healthy baby boy. However, her condition worsened postpartum as she developed high fever accompanied by bilateral lower limb paralysis and weakness along with loss of voluntary control over urination and defecation. The patient underwent ano-ther course of treatment consisting of steroids and IVIG; however there was limited improvement in symptoms observed after this intervention. Following administration of rituximab for the first time, the patient developed urinary tract infection which was successfully managed before continuing regular infusions. In later stages the patient could walk slightly with a limp and regained control over urination and defecation, allowing her to resume normal activities. This case suggests that combination therapy with steroids, IVIG, and hydroxychloroquine should be considered for the patients with pregnancy-associated NMOSD combined with Sjögren\'s syndrome. Rituximab can significantly improve symptoms such as postpartum paralysis in patients with NMOSD, however, there may be a risk of infection associated with its use.
摘要：
原发性干燥综合征（pSS）的中枢神经系统受累较少见，通常表现为白质病变。视神经脊髓炎谱系障碍（NMOSD），或者横贯性脊髓炎.NMOSD是一种免疫介导的中枢神经系统炎症性脱髓鞘疾病，复发率高，致残严重。研究表明，pSS合并NMOSD患者症状更严重，预后更差。这里,我们介绍了一例妊娠相关NMOSD合并干燥综合征的危重病病例。患者是一名30岁的孕妇，有干燥综合征病史，被诊断患有NMOSD。她接受了类固醇联合治疗,静脉注射免疫球蛋白（IVIG），和羟氯喹在怀孕期间，导致腰部以下麻木的部分分辨率。然而,由于医院以外的药物依从性不规律，她的右下肢出现了无力，伴有无法移动，而她的左下肢仍然有一些活动能力，但偶尔会麻木，并伴有尿失禁和大便失禁。十天后,她被送往急诊科，在那里进行了紧急剖腹产以分娩一名健康的男婴。然而,她的病情在产后恶化，因为她出现了高烧，并伴有双侧下肢瘫痪和无力，以及对排尿和排便的自愿控制。患者接受了由类固醇和IVIG组成的另一个疗程；然而，在这种干预后观察到的症状改善有限。首次服用利妥昔单抗后，患者出现尿路感染，在继续常规输注之前成功治疗。在后期，患者可以一瘸一拐地稍微行走，并重新控制排尿和排便，让她恢复正常活动.这个病例表明类固醇联合治疗,IVIG,与妊娠相关的NMOSD合并干燥综合征的患者应考虑使用羟氯喹。利妥昔单抗可以明显改善NMOSD患者的产后瘫痪等症状，然而,可能存在与使用相关的感染风险。
